Abstract

BackgroundRecurrent hemarthrosis that begin in childhood lead to progressive joint deterioration. Patients with haemophilia have chronic pain, functional disability and a reduced perception of health-related quality of life.PurposeTo analyse the perceived quality of life of adult patients with haemophilic arthropathy and its relationship with pain, joint condition, kinesiophobia and catastrophism.MethodsEighty-three adult patients with haemophilia were included in this multicentre, cross-sectional, descriptive study. Perceived quality of life (36-Item Short Form Health Survey), perceived usual and maximum pain (visual analogue scale), joint condition (Haemophilia Joint Health Score), kinesiophobia (Tampa Scale of Kinesiophobia) and catastrophism (Pain Catastrophizing Scale) were assessed. Sociodemographic, clinical and therapeutic variables and drug consumption for pain control were collected. Descriptive statistics used means and standard deviations. The correlation of quality of life with the dependent variables was calculated with the Pearson correlation test. The differences in quality of life as a function of the binomial variables were calculated with Student’s t-test for independent samples.ResultsPhysical component of quality of life perceived by patients with hemophilia is lower than Spanish population (30.51 VS 48.85). Regarding the mental component, patients with hemophilia showed higher values (56.07 VS 49.97). Catastrophism correlated (p < .05) with all items of quality of life questionnaire. Kinesiophobia correlated (p < .05) with all items of quality of life except to role-emotional (r = -.18; p > .05). Habitual and maximal joint pain correlated with all items except to role-emotional (r = − .19 and r = − .09, respectively) and mental component score (r = − .16 and r = − .07, respectively). Catastrophism and weekly drug intake were inversely correlated with quality of life. Age was positively correlated with perceived quality of life. There were differences in quality of life as a function of the severity of haemophilia and the intake of drugs for pain control.ConclusionsThe perceived quality of life of adult patients with haemophilia is worse than that of the Spanish population. Pain, kinesiophobia, catastrophism, haemophilia severity and the intake of pain-control medication influence the quality of life of these patients.

Highlights

  • Recurrent hemarthrosis that begin in childhood lead to progressive joint deterioration

  • Plain English summary This study identifies the clinical and therapeutic factors that affect the quality of life of adult patients with degenerative haemophilic arthropathy.The perceived quality of life of adult patients with joint damage and the factors that influence the quality of life of adult patients with haemophilic arthropathy were observed.A total of 83 patients with a rare disease and an advanced degree of joint damage were recruited from across the country

  • Catastrophism was negatively correlated with all domains of perceived quality of life (p < .05)

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Summary

Introduction

Recurrent hemarthrosis that begin in childhood lead to progressive joint deterioration. Patients with haemophilia have chronic pain, functional disability and a reduced perception of health-related quality of life. Purpose: To analyse the perceived quality of life of adult patients with haemophilic arthropathy and its relationship with pain, joint condition, kinesiophobia and catastrophism. In severe haemophilia, bleeding in the musculoskeletal system with mild or spontaneous trauma is common. Prophylactic treatment has been shown to be effective for reducing bleeding and its consequences [3], but because of its cost and associated discomfort, not all patients choose it [4]. The characteristic clinical manifestation of haemophilia is the development of musculoskeletal haemorrhages, mainly joint bleeding. Recurrent joint haemorrhagic episodes that begin in childhood lead to progressive joint deterioration [8], which causes changes in the cartilage, bone, synovium and vessels. In the final phase of joint damage, a degenerative and chronic lesion known as haemophilic arthropathy is established

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